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1.
Audiol Neurootol ; 2024 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-38527427

RESUMO

INTRODUCTION: Auditory performance in noise of cochlear implant recipients can be assessed with the adaptive Matrix test (MT); however, when the speech-to-noise ratio (SNR) exceeds 15 dB, the background noise has any negative impact on the speech recognition. Here, we aim to evaluate the predictive power of aided pure-tone audiometry and speech recognition in quiet, and establish cut-off values for both tests that indicate whether auditory performance in noise can be assessed using the Matrix sentence test in a diffuse noise environment. METHODS: Here, we assessed the power of pure-tone audiometry and speech recognition in quiet to predict the response to the MT. Ninety-eight cochlear implant recipients were assessed using different sound processors from Advanced Bionics (n=56) and Cochlear (n=42). Auditory tests were performed at least 1-year after cochlear implantation or upgrading the sound processor to ensure the best benefit of the implant. Auditory assessment of the implanted ear in free-field conditions included: pure-tone average (PTA), speech discrimination score (SDS) in quiet at 65 dB, and speech recognition threshold (SRT) in noise that is the SNR at which the patient can correctly recognize 50% of the words using the MT in a diffuse sound field. RESULTS: The SRT in noise was determined in sixty patients (61%) and undetermined in 38 (39%) using the MT. When cut-off values for PTA <36 dB and SDS >41% were used separately, they were able to predict a positive response to the MT in 83% of recipients; using both cut-off values together, the predictive value reached 92%. DISCUSSION/CONCLUSION: As the pure-tone audiometry is standardized universally and the speech recognition in quiet could vary depending on the language used; we propose that the MT should be performed in recipients with PTA <36 dB, and in recipients with PTA >36 dB, a list of Matrix sentences at a fixed SNR should be presented to determine the percentage of words understood. This approach should enable clinicians to obtain information about auditory performance in noise whenever possible.

2.
Artigo em Inglês | MEDLINE | ID: mdl-38194096

RESUMO

PURPOSE: This study aimed to investigate the effects of low frequency (LF) pitch perception on speech-in-noise and music perception performance by children with cochlear implants (CIC) and typical hearing (THC). Moreover, the relationships between speech-in-noise and music perception as well as the effects of demographic and audiological factors on present research outcomes were studied. METHODS: The sample consisted of 22 CIC and 20 THC (7-10 years). Harmonic intonation (HI) and disharmonic intonation (DI) tests were used to assess LF pitch perception. Speech perception in quiet (WRSq)/noise (WRSn + 10) were tested with the Italian bisyllabic words for pediatric populations. The Gordon test was used to evaluate music perception (rhythm, melody, harmony, and overall). RESULTS: CIC/THC performance comparisons for LF pitch, speech-in-noise, and all music measures except harmony revealed statistically significant differences with large effect sizes. For the CI group, HI showed statistically significant correlations with melody discrimination. Melody/total Gordon scores were significantly correlated with WRSn + 10. For the overall group, HI/DI showed significant correlations with all music perception measures and WRSn + 10. Hearing thresholds showed significant effects on HI/DI scores. Hearing thresholds and WRSn + 10 scores were significantly correlated; both revealed significant effects on all music perception scores. CI age had significant effects on WRSn + 10, harmony, and total Gordon scores (p < 0.05). CONCLUSION: Such findings confirmed the significant effects of LF pitch perception on complex listening performance. Significant speech-in-noise and music perception correlations were as promising as results from recent studies indicating significant positive effects of music training on speech-in-noise recognition in CIC.

3.
J Clin Med ; 12(20)2023 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-37892666

RESUMO

This PRISMA-compliant systematic review aimed to investigate the use of and the most common procedures performed with the novel 3D 4K exoscope in surgical pediatric head and neck settings. METHODS: Search criteria were applied to PubMed, EMBASE and the Cochrane Review databases and included all studies published up to January 2023 reporting 3D 4K exoscope-assisted surgeries in pediatric patients. After the removal of duplicates, selection of abstracts and full-text articles, and quality assessment, we reviewed eligible articles for number of patients treated, age, surgical procedures, and outcomes. RESULTS: Among 54 potentially relevant records, 5 studies were considered eligible and included in this systematic review, with reported treatment data for 182 patients. The surgical procedures belong to the otologic field (121 cases), head and neck surgery (25 cases) and transoral surgery (36 cases). Exoscopy allowed high quality visualization of anatomical structures during cochlear implantation and during reconstruction in head and neck surgery; moreover, it improved the surgical view of surgeons, spectators and ENT students. CONCLUSIONS: The use of 3D 4K exoscopy has shown promising potential as a valuable tool in pediatric ORL-head and neck surgery; nevertheless, further validation of these encouraging outcomes is necessary through larger-scale studies specifically focused on pediatric patients.

4.
Acta Otorhinolaryngol Ital ; 43(5): 352-359, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37519138

RESUMO

Objective: The COVID-19 pandemic profoundly modified the work routine in healthcare; however, its impact on the field of paediatric otorhinolaryngology (ORL) has been rarely investigated. The aim of this study was to assess the impact of COVID-19 on paediatric ORL. Methods: A questionnaire was developed by the Young Otolaryngologists of the Italian Society of ORL-Head and Neck Surgery (GOS). The questionnaire consisted of 26 questions related to workplace and personal paediatric ORL activities. The link was advertised on the official social media platforms and sent by e-mail to 469 Italian otolaryngologists. Results: The questionnaire was completed by 118 responders. During the pandemic, the main reduction was observed for surgical activity (78.8%), followed by outpatient service (16.9%). The conditions that were mostly impacted by a delayed diagnosis were respiratory infections in 45.8% of cases and sensorineural hearing loss in 37.3% of cases. Conclusions: Paediatric ORL was highly impacted by the COVID-19 pandemic, with a significant reduction of surgical and outpatient activities and a delay in time-sensitive diagnosis. Therefore, the implementation of new strategies, such as telemedicine, is recommended.

5.
Int Tinnitus J ; 26(1): 50-56, 2022 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-35861458

RESUMO

Meniere's Disease (MD) is an inner ear disorder characterized by spontaneous recurrent vertigo, fluctuating sensorineural hearing loss, aural fullness and low-pitch tinnitus. Therapeutic management of MD includes dietary restriction and medical therapy. A minority of cases is characterized by frequent vertigo attacks, progressive hearing loss and persistent tinnitus even through the continuous medical treatments; this condition is called intractable MD and requires a therapeutic escalation from non-invasive medical treatment to surgical intervention. Invasive procedures include endolymphatic sac surgery, vestibular nerve section and labyrinthectomy. These procedures have a very high success rate on symptom control but may have a severe impact on the hearing function. However, the simultaneous combined approach of demolitive surgery and cochlear implantation may be a valid approach to treat symptoms of intractable MD and preserve hearing function. In the present study, we review current literature focusing on intractable MD to describe and discuss advantages and disadvantages of established and newly proposed surgical treatments for intractable MD.


Assuntos
Implante Coclear , Perda Auditiva Neurossensorial , Doença de Meniere , Zumbido , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/cirurgia , Humanos , Doença de Meniere/diagnóstico , Doença de Meniere/cirurgia , Zumbido/etiologia , Zumbido/cirurgia , Vertigem
6.
Acta Otorhinolaryngol Ital ; 42(2): 169-175, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35612509

RESUMO

Objective: To analyse hearing attention and quality of listening in a cohort of children affected by moderate to severe unilateral hearing loss, comparing a group of hearing aid users to children with no hearing aid. Methods: Twenty-four children (12 hearing aid users, and 12 without hearing rehabilitation) underwent audiological evaluation with speech audiometry in quiet and noise, hearing attention tests, and the speech, spatial and quality of hearing questionnaire in the version for parents. Results: Concerning speech audiometry in noise, in the most difficult condition no one in the unaided group achieved a normal speech recognition threshold score (0% vs 11.6 ± 2.7% in the aided group). The selective hearing attention and shifting tests showed fewer errors in the aided group vs. the non-aided group. The questionnaire showed a significant difference between the quality of listening perceived by the parents of the two groups. The mean total scores were 152.8 ± 2.7 in the aided group and 116.1 ± 2.6 in the non-aided group (p > 0.001). Conclusions: Children with unilateral hearing loss without hearing rehabilitation show a lower quality of hearing, especially with regards to divided auditory attention. Hearing rehabilitation should be proposed as soon as possible in children with moderate to severe unilateral hearing loss.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Perda Auditiva Unilateral , Percepção da Fala , Atenção , Criança , Audição , Perda Auditiva Neurossensorial/reabilitação , Humanos
7.
Cochlear Implants Int ; 23(2): 70-79, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34844527

RESUMO

OBJECTIVE: Immune-mediated inner ear disease (IMIED) might cause severe/profound hearing loss and these patients are considered ideal candidates to cochlear implant (CI) surgery. The aim of the study was to evaluate impedance changes over time. METHOD: The Study Group (SG) was composed of CI IMIED patients (31 ears) and a Control Group (CG) of CI patients with hearing loss not related to their immune system (31 ears). Audiological performance and impedance values were measured and compared amongst groups at 3, 6, 12 and 18 months following the fitting sessions. RESULTS: Speech perception was significantly better for SG in word and sentence recognition in quiet. Impedance values were, on average, significantly higher for apical and middle electrode segments in SG compared to CG at the 3- month follow-up and were maintained over time. Additionally, a subset of SG patients (active patients) experienced significantly greater impedance fluctuation corresponding to clinical symptom reactivation. CONCLUSION: IMIED patients achieve good audiological performance. However, the relapsing inflammation could change the inner ear environment, causing impedance fluctuations and, consequently, more frequent CI fittings. Additionally, impedance evaluation could be utilized as an early warning sign of IMIED recurrence and as an aid to therapeutic decision-making.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva , Doenças do Labirinto , Percepção da Fala , Impedância Elétrica , Humanos , Percepção da Fala/fisiologia
8.
Eur Arch Otorhinolaryngol ; 278(1): 41-48, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32449024

RESUMO

PURPOSE: Binge drinking is associated with several adverse effects in multiple organs. This study aimed at evaluating the effects of a binge-like-drinking on the vestibulo-oculomotor reflex (VOR) using the video Head Impulse Test (vHIT) and the functional Head Impulse Test (fHIT). METHODS: Eleven healthy men (age range 32-35 years) with moderate drinking habits and no history of vestibular dysfunction were enrolled. A preliminary assessment of breath alcohol concentration (BrAC) to check for zero alcohol value and a pre-intake evaluation of VOR using the vHIT and the fHIT were carried on. Then, the subjects were asked to take drinks with different alcohol content (8-40% ethanol by volume) according to their choice, consuming at least 5 standard drinks. Volunteers stopped drinking after 3 h. After a further 30 min, post-intake BrAC measurements and VOR analysis were repeated. RESULTS: After alcohol intake, vHIT recorded an overall significant reduction of VOR gain (0.82 ± 0.07 on both sides) although the outcomes were below the normal range only in the four subjects with the highest blood alcohol levels. The post-intake fHIT outcomes were substandard in 9 participants, with a significant deterioration in performance (% of exact answers = 84.54 ± 11.05% on the left, 83.18 ± 14.53 on the right). CONCLUSIONS: Binge drinking severely affects VOR; fHIT seems more sensitive than vHIT in the assessment of VOR function for complex vestibular lesions, such as those determined by ethanol, suggesting that fHIT could support vHIT in vestibular dysfunction assessment.


Assuntos
Consumo de Bebidas Alcoólicas/efeitos adversos , Consumo Excessivo de Bebidas Alcoólicas , Teste do Impulso da Cabeça/métodos , Reflexo Vestíbulo-Ocular/efeitos dos fármacos , Doenças Vestibulares/diagnóstico , Adulto , Etanol , Humanos , Masculino , Reflexo Vestíbulo-Ocular/fisiologia , Doenças Vestibulares/fisiopatologia , Vestíbulo do Labirinto , Gravação em Vídeo
9.
Ear Nose Throat J ; 100(1_suppl): 51S-58S, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32511005

RESUMO

INTRODUCTION: The aim of the current systematic review is to update the pooled survival outcome of patients with T2 glottic carcinoma treated with either laser surgery (CO2 transoral laser microsurgery [CO2 TOLMS]), radiotherapy (RT), or open partial laryngectomy (OPL). METHODS: A systematic search was performed using the MEDLINE database, Scopus, and Google scholar. The inclusion criteria were studies of patients with T2N0 glottic tumor, treated with either primary CO2 TOLMS, definitive curative RT, or primary OPL, and with reported oncological outcome at 5 years calculated with a Kaplan-Meier or Cox regression method. RESULTS: The results of the current review show that local control (LC) is higher with OPL 94.4%, while there are no differences in LC at 5-year posttreatment for patients treated with RT, compared to those treated with CO2 TOLMS (respectively, 75.6% and 75.4%). Primary treatment with OPL and CO2 TOLMS results in higher laryngeal preservation than primary treatment with RT (respectively 95.8%, 86.9%, and 82.4%). CONCLUSION: First-line treatment with OPL and CO2 TOLMS should be encouraged in selected T2 patients, because it results in higher laryngeal preservation and similar LC compared to primary treatment with RT. The involvement of the anterior commissure in the craniocaudal plane and T2b impaired vocal cord mobility have a poorer prognosis and LC compared to patients with T2a tumors for both CO2 TOLMS and RT.


Assuntos
Carcinoma/terapia , Neoplasias Laríngeas/terapia , Laringectomia/mortalidade , Terapia a Laser/mortalidade , Microcirurgia/mortalidade , Radioterapia/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma/mortalidade , Carcinoma/patologia , Intervalo Livre de Doença , Feminino , Glote/efeitos da radiação , Glote/cirurgia , Humanos , Estimativa de Kaplan-Meier , Neoplasias Laríngeas/mortalidade , Neoplasias Laríngeas/patologia , Laringectomia/métodos , Laringe/efeitos da radiação , Laringe/cirurgia , Terapia a Laser/métodos , Lasers de Gás/uso terapêutico , Masculino , Microcirurgia/métodos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Modelos de Riscos Proporcionais , Radioterapia/métodos , Resultado do Tratamento , Prega Vocal/efeitos da radiação , Prega Vocal/cirurgia
10.
J Neuroimaging ; 31(1): 90-97, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33146926

RESUMO

BACKGROUND AND PURPOSE: Using functional magnetic resonance imaging (fMRI), we explored cortical activation in patients with acute Bell's palsy (BP) and analyzed its correlates with clinical status in the acute phase, and with 6-month outcome. METHODS: Twenty-four right-handed patients with acute BP within 15 days of onset and 24 healthy controls underwent fMRI during performance of unilateral active (hemi-smiling) and passive lip movement tasks with both the paretic and the normal lip. The degree of paresis was evaluated during the acute stage and at the 6-month follow up using the House-Brackmann (HB) grading scale. Complete recovery was defined as HB grade II or less at the end of the 6-month period. The difference in the HB grade (ΔHB) between the acute stage and the 6-month follow up was used to evaluate clinical improvement. RESULTS: There were 24 patients with unilateral acute BP. HB grades ranged from III to VI. At 6 months, 11 patients (46%) had completely recovered and 12 (50%) were partially improved. Compared with healthy subjects, BP patients had a significantly greater activation of the frontal areas and the insula ipsilateral to the paretic side. In BP patients, there was an inverse correlation between the activation of the ipsilateral hemisphere when moving the paretic side and the degree of paresis at baseline. An association was also observed between activation and clinical outcome (both complete recovery and ΔHB). CONCLUSIONS: In patients with BP, fMRI may represent a useful tool to predict long-term outcome, guide therapeutic approach, and monitor treatment response.


Assuntos
Paralisia de Bell/diagnóstico por imagem , Imageamento por Ressonância Magnética , Doença Aguda , Adulto , Paralisia de Bell/complicações , Paralisia de Bell/fisiopatologia , Estudos de Casos e Controles , Humanos , Masculino , Pessoa de Meia-Idade , Movimento , Paresia/complicações , Adulto Jovem
12.
Int J Audiol ; 60(6): 469-478, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33174776

RESUMO

OBJECTIVE: Bimodal stimulation is a standard option for asymmetric hearing loss in adults. Questions have been raised whether receiving two stimulations may conflict in elderly listeners where the central integration of an acoustic/electrical signal may be very important to obtain benefit in terms of speech perception. DESIGN: Clinical retrospective study. STUDY SAMPLE: The outcomes from 17 bimodal cochlear implant (CI) users were analysed. The test material consisted of speech audiometry in quiet and in noise (STARR and Matrix). RESULTS: Bimodal PTA and speech perception both in quiet and in noise were significantly better than CI or HA alone. Age showed a significant effect on bimodal STARR outcomes. Similarly, bimodal STARR scores improved significantly in comparison to Better Ear. CONCLUSION: Both Matrix and STARR tests were very difficult for many elderly CI listeners from the present study group, especially in unilateral listening condition. The performance improved significantly, emphasising a good integration of acoustic and electric hearing in this group of elderly bimodal listeners. Overall results highlighted how a specific study, based on speech perception in noise in the elderly listeners, might shed light on the effect of speech test modality on bimodal outcomes.


Assuntos
Implante Coclear , Implantes Cocleares , Auxiliares de Audição , Perda Auditiva , Percepção da Fala , Adulto , Idoso , Humanos , Estudos Retrospectivos
13.
Front Neurosci ; 14: 556675, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33240035

RESUMO

OBJECTIVE: The aim of the present study was to investigate the pupillary response to word identification in cochlear implant (CI) patients. Authors hypothesized that when task difficulty (i.e., addition of background noise) increased, pupil dilation markers such as the peak dilation or the latency of the peak dilation would increase in CI users, as already observed in normal-hearing and hearing-impaired subjects. METHODS: Pupillometric measures in 10 CI patients were combined to standard speech recognition scores used to evaluate CI outcomes, namely, speech audiometry in quiet and in noise at +10 dB signal-to-noise ratio (SNR). The main outcome measures of pupillometry were mean pupil dilation, maximal pupil dilation, dilation latency, and mean dilation during return to baseline or retention interval. Subjective hearing quality was evaluated by means of one self-reported fatigue questionnaire, and the Speech, Spatial, and Qualities (SSQ) of Hearing scale. RESULTS: All pupil dilation data were transformed to percent change in event-related pupil dilation (ERPD, %). Analyses show that the peak amplitudes for both mean pupil dilation and maximal pupil dilation were higher during the speech-in-noise test. Mean peak dilation was measured at 3.47 ± 2.29% noise vs. 2.19 ± 2.46 in quiet and maximal peak value was detected at 9.17 ± 3.25% in noise vs. 8.72 ± 2.93% in quiet. Concerning the questionnaires, the mean pupil dilation during the retention interval was significantly correlated with the spatial subscale score of the SSQ Hearing scale [r(8) = -0.84, p = 0.0023], and with the global score [r(8) = -0.78, p = 0.0018]. CONCLUSION: The analysis of pupillometric traces, obtained during speech audiometry in quiet and in noise in CI users, provided interesting information about the different processes engaged in this task. Pupillometric measures could be indicative of listening difficulty, phoneme intelligibility, and were correlated with general hearing experience as evaluated by the SSQ of Hearing scale. These preliminary results show that pupillometry constitutes a promising tool to improve objective quantification of CI performance in clinical settings.

14.
Artigo em Inglês | MEDLINE | ID: mdl-32349379

RESUMO

The association between hyperacusis and developmental disorders such as autism spectrum disorders has been extensively reported in the literature; however, the specific prevalence of hyperacusis in attention deficit hyperactivity disorder (ADHD) has never been investigated. In this preliminary study, we evaluated the presence of hyperacusis in a small sample of children affected by ADHD compared to a control group of healthy children. Thirty normal hearing children with a diagnosis of ADHD and 30 children matched for sex and age were enrolled in the study. All children underwent audiological and multidisciplinary neuropsychiatric evaluation. Hearing was assessed using pure tone audiometry and immittance test; ADHD was diagnosed following the Diagnostic and Statistical Manual of Mental Disorder criteria. Hyperacusis was assessed through the administration of a questionnaire to parents and an interview with children. Hyperacusis was diagnosed in 11 children (36.7%) in the study group and in four children (13.3%) in the control group; this difference was statistically significant (p = 0.03). The preliminary results of this study suggest a higher presence of hyperacusis in children with attention deficit hyperactivity disorder compared to control children. More studies on larger samples are necessary to confirm these results.


Assuntos
Transtorno do Deficit de Atenção com Hiperatividade , Hiperacusia , Transtorno do Deficit de Atenção com Hiperatividade/complicações , Estudos de Casos e Controles , Criança , Manual Diagnóstico e Estatístico de Transtornos Mentais , Humanos , Hiperacusia/complicações , Inquéritos e Questionários
15.
Neuroscience ; 436: 136-153, 2020 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-32278721

RESUMO

Unilateral noise-induced hearing loss reduces the input to the central auditory pathway disrupting the excitatory and inhibitory inputs to the inferior colliculus (IC), an important binaural processing center. Little is known about the compensatory synaptic changes that occur in the IC as a consequence of unilateral noise-induced hearing loss. To address this issue, Sprague-Dawley rats underwent unilateral noise exposure resulting in severe unilateral hearing loss. IC tissues from the contralateral and ipsilateral IC were evaluated for acute (2-d) and chronic (28-d) changes in the expression of 84 synaptic plasticity genes on a PCR array. Arc and Egr1 genes were further visualized by in situ hybridization to validate the PCR results. None of the genes were upregulated, but many were downregulated post-exposure. At 2-d post-exposure, more than 75% of the genes were significantly downregulated in the contralateral IC, while only two were downregulated in the ipsilateral IC. Many of the downregulated genes were related to long-term depression, long-term potentiation, cell adhesion, immediate early genes, neural receptors and postsynaptic density. At 28-d post-exposure, the gene expression pattern was reversed with more than 85% of genes in the ipsilateral IC now downregulated. Most genes previously downregulated in the contralateral IC 2-d post-exposure had recovered; less than 15% remained downregulated. These time-dependent, asymmetric changes in synaptic plasticity gene expression could shed new light on the perceptual deficits associated with unilateral hearing loss and the dynamic structural and functional changes that occur in the IC days and months following unilateral noise-induced hearing loss.


Assuntos
Perda Auditiva Provocada por Ruído , Colículos Inferiores , Estimulação Acústica , Animais , Vias Auditivas , Plasticidade Neuronal , Ratos , Ratos Sprague-Dawley
16.
Ital J Pediatr ; 46(1): 3, 2020 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-31906974

RESUMO

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is the most frequent non-genetic cause of sensorineural hearing-loss (SNHL) (i.e., hearing loss due to a cochlear and/or auditory nerve damage). It is widely accepted that SNHL at birth, when associated to cCMV symptomatic infection involving the central nervous system, benefits from antiviral therapy started in the neonatal period. Conversely, there is no consensus for antiviral treatment in congenitally infected infants diagnosed with isolated SNHL (i.e., SNHL in an otherwise asymptomatic infant) at birth. Our aim was to assess the frequency and the auditory outcome of isolated SNHL at birth due to auditory neuropathy (AN) (i.e., SNHL in a patient with normal cochlear function and auditory nerve dysfunction) in infants with cCMV infection. METHODS: We retrospectively reviewed the clinical history of 60 infants, born at term, with cCMV asymptomatic infection, without additional risk factors for SNHL, and exhibiting bilateral "pass" otoacustic emissions (OAE). None of them underwent antiviral therapy. Hearing thresholds were assessed by means of Auditory Brainstem Responses (ABR). AN affected children were followed up until possible normalization of the hearing thresholds or definitive diagnosis of AN. Each infant diagnosed with monolateral or bilateral AN was classified according to the worst ear threshold. RESULTS: In our population, the first ABR was performed at a mean age of 5.00 ± 2.79 (SD) months and AN was diagnosed in 16/60 (26.67%) infants; in 4 infants the AN was defined as mild (4/4 monolateral), moderate in 11 (5/11 bilateral), and severe in 1 (bilateral). The mean age at first ABR was 3.69 ± 2.80 (SD) months in the 16 babies with AN and 5.48 ± 2.66 (SD) months in the 44 infants with normal hearing (p = 0.007). All AN cases spontaneously recovered a normal auditory threshold over time. The mean length of the audiological follow-up was 32.44 ± 17.58 (SD) months (range 5-60 months). CONCLUSION: A delayed maturation of the auditory pathways should be considered when a mild/moderate isolated AN at birth is detected in cCMV infected infants. Prospective studies conducted on larger populations, and with a longer audiological follow-up, are needed to confirm our findings.


Assuntos
Infecções por Citomegalovirus/congênito , Perda Auditiva Central/virologia , Potenciais Evocados Auditivos do Tronco Encefálico , Feminino , Perda Auditiva Neurossensorial/virologia , Testes Auditivos , Humanos , Lactente , Recém-Nascido , Itália , Masculino , Triagem Neonatal , Estudos Retrospectivos
17.
Clin Rev Allergy Immunol ; 59(3): 304-322, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31376044

RESUMO

Amyloidosis constitutes a large spectrum of diseases characterized by an extracellular deposition of a fibrillar aggregate, generating insoluble and toxic amasses that may be deposited in tissues in bundles with an abnormal cross-ß-sheet conformation, known as amyloid. Amyloid may lead to a cell damage and an impairment of organ function. Several different proteins are recognized as able to produce amyloid fibrils with a different tissue tropism related to the molecular structure. The deposition of amyloid may occur as a consequence of the presence of an abnormal protein, caused by high plasma levels of a normal protein, or as a result of the aging process along with some environmental factors. Although amyloidosis is rare, amyloid deposits play a role in several conditions as degenerative diseases. Thus, the development of antiamyloid curative treatments may be a rational approach to treat neurodegenerative conditions like Alzheimer's disease in the future. Nowadays, novel treatment options are currently refined through controlled trials, as new drug targets and different therapeutic approaches have been identified and validated through modern advances in basic research. Fibril formation stabilizers, proteasome inhibitors, and immunotherapy revealed promising results in improving the outcomes of patients with systemic amyloidosis, and these novel algorithms will be effectively combined with current treatments based on chemotherapeutic regimens. The aim of this review is to provide an update on diagnosis and treatment for systemic amyloidosis.


Assuntos
Amiloidose/diagnóstico , Amiloidose/etiologia , Amiloidose/terapia , Proteínas Amiloidogênicas/genética , Proteínas Amiloidogênicas/metabolismo , Amiloidose/epidemiologia , Biópsia , Terapia Combinada , Diagnóstico por Imagem , Gerenciamento Clínico , Suscetibilidade a Doenças , Humanos , Rim/metabolismo , Rim/patologia , Especificidade de Órgãos , Avaliação de Sintomas
18.
Immunol Res ; 66(6): 675-685, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30270399

RESUMO

The role of the immune system in mediating cochleovestibular pathologies has received increasing attention in recent years. Autoimmune vertigo may be an invalidating condition and may worsen the quality of life of affected patients, especially in the cases of delayed diagnosis. Since the etiopathogenesis is still not clear, also the treatment is not yet completely delineated. According to the clinical presentation, autoimmune vertigo can present as an isolated disorder or in association with systemic autoimmune diseases. The main feature in autoimmune vertigo is the presence of an abnormal immune response, in either absence or presence of systemic autoimmune disease, directed against delicate components of the inner ear. This may determine a functional or anatomical alteration, with an inflammatory reaction often devastating for hearing and balance. Being the exact pathogenesis unknown, the diagnosis of autoimmune vertigo is based either on clinical criteria or on a positive response to steroids. The earlier the diagnosis is made, the sooner the therapy can be installed, giving a chance to the recovery of inner ear damages. Corticosteroids represent the most effective and universally accepted treatment, even if other immunomodulatory drugs are now having a more extensive use. HIGHLIGHTS: Vertigo is relatively frequent in autoimmune diseases; however, it is often misdiagnosed or attributed to central nervous system alterations rather to specific inner ear involvement. Vertigo and other audiovestibular symptoms may be the first manifestation of an autoimmune disease and if correctly addressed could significantly contribute to early diagnosis of the underlying autoimmune disease. Early diagnosis of immune-related vertigo can lead to prompt initiation of targeted therapy with elevate chances of preventing irreversible damages to the inner ear. The presence of alternating phases of well-being and disabling symptoms in patients with vertigo should always been considered, as they could suggest an underlying autoimmune condition.


Assuntos
Doenças Autoimunes/imunologia , Vertigem/imunologia , Doenças Vestibulares/imunologia , Corticosteroides/farmacologia , Animais , Doenças Autoimunes/tratamento farmacológico , Autoimunidade/efeitos dos fármacos , Autoimunidade/imunologia , Orelha Interna/imunologia , Humanos , Vertigem/tratamento farmacológico , Doenças Vestibulares/tratamento farmacológico
19.
Ital J Pediatr ; 44(1): 104, 2018 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-30143030

RESUMO

BACKGROUND: The introduction of Universal Newborn Hearing Screening (UNHS) programs has drastically contributed to the early diagnosis of hearing loss in children, allowing prompt intervention with significant results on speech and language development in affected children. UNHS in the Lazio region has been initially deliberated in 2012; however, the program has been performed on a universal basis only from 2015. The aim of this retrospective study is to present and discuss the preliminary results of the UNHS program in the Lazio region for the year 2016, highlighting the strengths and weaknesses of the program. METHODS: Data from screening facilities in the Lazio region for year 2016 were retrospectively analyzed. Data for Level I centers were supplied by the Lazio regional offices; data for Level II and III centers were provided by units that participated to the study. RESULTS: During 2016, a total of 44,805 babies were born in the Lazio region. First stage screening was performed on 41,821 children in 37 different birth centers, with a coverage rate of 93.3%. Of these, 38.977 (93.2%) obtained a "pass" response; children with a "refer" result in at least one ear were 2844 (6.8%). Data from Level II facilities are incomplete due to missing reporting, one of the key issues in Lazio UNHS. Third stage evaluation was performed on 365 children in the three level III centers of the region, allowing identification of 70 children with unilateral (40%) or bilateral (60%) hearing loss, with a prevalence of 1.6/1000. CONCLUSIONS: The analysis of 2016 UNHS in the Lazio region allowed identification of several strengths and weaknesses of the initial phase of the program. The strengths include a correct spread and monitoring of UNHS among Level I facilities, with an adequate coverage rate, and the proper execution of audiological monitoring and diagnosis among Level III facilities. Weakness, instead, mainly consisted in lack of an efficient and automated central process for collecting, monitoring and reporting of data and information.


Assuntos
Diagnóstico Precoce , Perda Auditiva/diagnóstico , Testes Auditivos/métodos , Triagem Neonatal/métodos , Estudos de Coortes , Feminino , Seguimentos , Perda Auditiva/congênito , Perda Auditiva/epidemiologia , Humanos , Recém-Nascido , Itália/epidemiologia , Masculino , Prevalência , Estudos Retrospectivos , Medição de Risco , População Rural
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